Geographical variation in the clinical presentation of endomyocardial fibrosis in India?

OBJECTIVES: To compare the clinical presentation, clinical profile and survival of two groups of endomyocardial fibrosis patients. METHODS: The study was a prospective cohort study, or a prospective case series, comparing all consecutive echocardiographically proven patients with endomyocardial fibrosis seen in Medical College Trivandrum with the patients seen in Medical College Hospital, Alappuzha(Alleppey) (or TD Medical College). In all patients the clinical details like age, sex, type of endomyocardial fibrosis, the presence of anaemia, eosinophilia, neutrophilia and type of rhythm(Sinus or atrial fibrillation) etc were compared by both simple X2 and by Kaplan Meier survival curves. RESULTS: The mean age and the sex distribution was same in both places Briefly the incidence of biventricular endomyocardial fibrosis was more from Trivandrum than Alleppey, 64.9% vs 14.3% (p<0.0.001), the incidence of atrial fibrillation was more in Trivandrum 44.2% vs 16.3%. (p<0.001)The overall survival of Trivandrum patients was poorer (p<0.0001). The six year survival was 61% in the Trivandrum population whereas it was 91.5% in the Alleppey population. CONCLUSIONS: These differences may have been due to the better nutrition of the Alleppey patients due to a higher exposure to fish compared to the Trivandrum population. Better nutrition would protect against Magnesium deficiency and prevent the absorption of Cerium in the patients from Alleppey, compared to those from Trivandrum.

A prominent 'A' notch in the pulmonary valve M mode-one more cause of the same.

The M mode echocardiogram is not the main priority, during routine echocardiographic evaluation now-a-days. However, there are still a few classical conditions where this remains instructive and educative. One such situation is the presence of an 'a' wave in the pulmonary valve M mode tracing in normals and it's absence in pulmonary hypertension. In valvular pulmonary stenosis we expect a deeper 'a' wave. We describe one more cause of a prominent 'a' wave in the pulmonary valve M mode. We describe this in a common tropical disease, endemic to Kerala.

A study of ECG pattern, cardiac structural abnormalities and familial tendency in patients with early repolarisation syndrome in South India.

BACKGROUND: Early repolarisation (ER) on ECG, which was initially believed to be benign, has of late been considered otherwise. Brugada syndrome has recently been thought to be an extension of the ER spectrum, and the familial tendency of the ER pattern is being highlighted. With attention being drawn to ER's association with idiopathic ventricular fibrillation (VF), the prognosis and lineage of patients with an ER pattern are under scrutiny. AIMS: To analyse ER patterns on ECG, their presence in first-degree relatives and their association with structural heart disease. To classify different types of ER and estimate the prevalence of the high-risk notch/slur pattern in the population studied. METHODS: We screened all patients presenting to our department from December 2011 to July 2014 for ER patterns. We excluded patients with other causes of ST elevation that mimicked the ER pattern, those aged <18 years, and those not willing to participate in the study. A complete physical examination, 12-lead ECG and echocardiography were performed on all study patients. Willing first-degree relatives were screened with a 12-lead ECG. Of the 963 patients with ER that we initially screened, 843 completed the study. A total of 4116 relatives were screened. RESULTS: Of the 843 patients who completed the study, 687 (81.5%) were male and 156 (18.5%) were female. The majority were asymptomatic (70.11%), but had been referred for ECG abnormalities. Fifteen patients with chest pain were inadvertently thrombolysed and were later diagnosed to have ER. Their ER pattern was exaggerated during chest pain, which made this error highly likely. Among the 48 patients who had acute coronary syndrome (ACS), ER pattern was noticed in a different lead than those affected by ACS. Of these, 27 (56.25%) had ventricular tachycardia/VF during the acute phase. Six patients had electrical storm without evidence of ACS, and all had a global ER pattern with prominent notching/slurring on baseline ECG. The most common type of ER pattern was type I (lateral leads; 55.87%). Twenty-one patients had a Brugada pattern. Of all the patients with ER, only a third (34.16%) had the possibly high-risk notched/slurred ECG pattern. The majority (82.92%) had a structurally normal heart. We found that mitral valve prolapse (MVP), as assessed by >2 mm leaflet prolapse from the annulus, was more common in patients with ER (11.39%). Of the 4116 relatives screened, 2625 (63.78%) had an ER pattern; a quarter of family members had the inferolateral variety and over 60% of relatives had the lateral variety. We also noticed different ER patterns in the same family. CONCLUSIONS: We found that exaggeration of the ER pattern during chest pain may lead to inadvertent thrombolysis. A notched/slurred ER pattern is found in only a third of patients, who need to be grouped separately, as they may constitute a high-risk category. Patients with ER had MVP at a higher prevalence (almost double) than the general population, probably explaining the high incidence of sudden cardiac death associated with MVP. A familial tendency to an ER pattern was found in more than half of first-degree relatives, with different ER patterns, even the Brugada pattern, found in the same family. This may be because Brugada and other ER patterns belong to the same spectrum and may share the same prognosis. Thus we conclude that further studies regarding ER, its association with MVP, risk stratification by notched ECG pattern, and familial distribution along with gene analysis are warranted.

An uncommon picture of endomyocardial fibrosis: no embolism yet.

We present here a review of the various gradings of endomyocardial fibrosis from autopsy (Shaper's types). Echocardiography accurately delineates the extent of fibrosis of either the right or left ventricle and we have illustrated a typical classical case. We have images of the same patient from 2010, 2011 and 2012 and so we use this to illustrate the echocardiographic gradings.